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1.Infantile Choledochal Cyst Presenting with Neonatal Cholestasis; Review of Anatomical and Clinical Aspect.

Ulcerative colitis, one of the inflammatory bowel disease, is primarily managed medically with combinations of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, the indications and outcome for surgical management of pediatric ulcerative colitis patients were studied from medical records retrospectively. Twenty-one patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were subjected to the study. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management primarily after diagnosis and 8 patients (38%) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53%) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure owing to sclerosing cholangitis respectively. In 8 patients who received surgical management, 1 patient underwent surgery due to sigmoid colon perforation and 7 patients due to intractability of medical management. The perforated case received colon segmental resection and the other 7 patients recieved total protocolectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. One patient is still on medical management because of mild persistent hematochezia after surgery. The other operated patients are showing good prognosis without any management. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation is present. Total protocolectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.
Child* ; Cholangitis, Sclerosing ; Chronic Disease ; Colitis, Ulcerative* ; Colon ; Colon, Sigmoid ; Diagnosis ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; Humans ; Inflammatory Bowel Diseases ; Liver Failure ; Medical Records ; Pneumonia ; Prognosis ; Retrospective Studies ; Seoul ; Sepsis ; Steroids ; Ulcer*

This study is based on 155 patients of congenital me colon. For the diagnosis, 93 cases were histologically proven and the remaining 63 cases were diagnosed on clinical basis including barium enema or surgical gross findings. On histologic examination, 80 cases(86%) showed typical features of absence of ganglion cell in the myenteric plexus and the 13 cases(14%) had atypical features which were segmental absence Of ganglion cell in one case. There we 127 males(82%) and 28 females(18%). The age at diagnosis was younger than 30 days in 87 cases(56%), I month to 1 year in 39 cases(25%) and older than I year in 29 cases(18%). The levels of aganglionosis were variable: short segment (rectosigmoid) in 134 cases(86%), intermediate segment (more proximal colon) in 14 cases(100%). and 6 cases(4%) had total aganglionosis. Common clinical presentations were abdominal distention, delayed meconium passage or bilious vomiting in neonate, and chronic constipation in infancy or childhood. Following initial colostomy or ileostomy, a definitive procedure was performed in 151 cases(Duhamel type in 150 cases; Soave type in 2 cases; Swenson type in 3 cases). Frequently associated problems after definitive procedure were persistent constipation(ll%) due to septum formation, fecaloma, remnant aganglionic segment and rectal stenosis. Overall mortality rate was 4%, and increased mortality was associated with enterocolitis(14%) which was the most frequent cause of death. The follow-up study longer than 3 months was available in 138 patients who underwent a definitive procedure(mean 2 year 11 months). Seventy-three cases(53%) had normal bowel function, 38cases(27.5%) had occasionally used enema or stool softners, and 27 cases(19.5%) had severe constipation or soiling. The bowel habit improved with time, and were considered normal in 60% of patients after follow-up more than 3 years. The results of definitive procedures for congenital megacolon including Duhamel operation was satisfactory, and long-term follow-up appeared an important and critical component of patients'care.
Child ; Male ; Female ; Infant, Newborn ; Humans ; Mortality ; Follow-Up Studies